Expansion of a GGGGCC (G4C2) hexanucleotide repeat sequence in the non-coding region of human chromosome 9 open reading frame 72 or C9orf72 (also known as ALSFTD, FTDALS; Gene ID 203228) is the most common genetic abnormality in familial and sporadic frontotemporal dementia (FTD) and motor neuron disease (MND), with amyotrophic lateral sclerosis (ALS) as the most frequent form. The number of hexanucleotide repeats in the normal population ranges from 2 to 24, whereas up to several thousand repeats (700 - 4,400 repeats) are found in the pathologically expanded allele. Unconventional repeat-associated non-ATG-initiated translation (RANT) of the GGGGCC repeats in the three alternate reading frames from both sense and antisense transcripts generates five types of dipeptide repeat (DPR) protein species, each composed of repeating units of two amino acids, glycine-alanine (GA), glycine-proline (GP), glycine-arginine (GR), alanine-proline (AP), proline-arginine (PR), respectively. The sense transcript (5′-GGGGCC-3′ hexanucleotide repeats) is translated through repeat-associated non-ATG (RAN) translation such that poly- (Gly- Ala), poly-(Gly-Pro), and poly-(Gly-Arg) proteins were produced. The anti-sense transcript (containing 5′-GGCCCC-3′ (G2C4) hexanucleotide repeats is translated through repeat-associated non-ATG (RAN) translation producing poly-(Pro-Ala), poly- (Pro- Arg), poly- (Gly- Pro) proteins. The antisense proteins accumulate in cytoplasmic aggregates in affected brain regions, including the frontal and motor cortex, hippocampus, and spinal cord neurons.
This polyclonal antibody recognizes C9ORF72/C9RANT (poly-PR).
Anti-C9ORF72/C9RANT (Poly-PR), Cat. No. ABN1354, is a highly specific rabbit polyclonal antibody, that targets C9ORF72 (alpha-PR antisense) and has been tested in Dot Blot, Immunofluorescence, Immunohistochemistry, and Western Blotting.
Immunofluorescence Analysis: A 1:500 dilution from a representative lot detected C9ORF72/C9RANT (Poly-PR) in HEK293Transfected Flag-PR. (Courtesy of Dr. Laura P.W. Ranum and Dr. Lien Nguyen from the University of Florida).
Immunofluorescence Analysis: A representative lot detected C9ORF72/C9RANT (Poly-PR) in transfected HEK293T Flag-PA cells (Zu, T., et. al. (2013). Proc. Natl. Acad. Sci. USA. 110(51):E4968-77).
Western Blotting Analysis: A representative lot detected C9ORF72/C9RANT (Poly-PR) in recombinant Protein (Zu, T., et. al. (2013). Proc. Natl. Acad. Sci. USA. 110(51):E4968-77).
Immunohistochemistry Analysis: A representative lot detected C9ORF72/C9RANT (Poly-PR) in aggregates found in neurons of the CA and DG regions of the hippocampus (Zu, T., et. al. (2013). Proc. Natl. Acad. Sci. USA. 110(51):E4968-77).
Dot Blotting Analysis: A representative lot detected C9ORF72/C9RANT (PR-CT) in Dot Blotting applications (Zu, T., et. al. (2013). Proc Natl Acad Sci USA. 110(51):E4968-77).
Evaluated by Western Blotting in lysate from Flag-PR transfected HEK293 cells.
Western Blotting Analysis: A 1:1,000 dilution of this antibody detected C9ORF72/C9RANT (Poly-PR) in 10 µL of lysate from HEK293 cells transfected with Flag-PR.
~18 kDa observed. Uncharacterized bands may be observed in some lysate(s).
Rabbit polyclonal serum with 0.05% sodium azide.
Storage and Stability
Stable for 1 year at -20°C from date of receipt. Handling Recommendations: Upon receipt and prior to removing the cap, centrifuge the vial and gently mix the solution. Aliquot into microcentrifuge tubes and store at -20°C. Avoid repeated freeze/thaw cycles, which may damage IgG and affect product performance.
Concentration: Please refer to lot specific datasheet.
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